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| | NDUFA8 | NADH:ubiquinone oxidoreductase subunit A8. (244 aa) |
| | EFHD1 | Uncharacterized protein. (235 aa) |
| | NDUFS1 | NADH:ubiquinone oxidoreductase core subunit S1; Belongs to the complex I 75 kDa subunit family. (741 aa) |
| | TMEM11 | Transmembrane protein 11, mitochondrial. (192 aa) |
| | LYRM7 | LYR motif containing 7; Belongs to the complex I LYR family. (105 aa) |
| | ACAD8 | Isobutyryl-CoA dehydrogenase, mitochondrial. (415 aa) |
| | ALDH1L2 | Aldehyde dehydrogenase 1 family member L2; Belongs to the aldehyde dehydrogenase family. (941 aa) |
| | SDHA | Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial; Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). (664 aa) |
| | NDUFA6 | NADH:ubiquinone oxidoreductase subunit A6; Belongs to the complex I LYR family. (154 aa) |
| | CBR4 | Carbonyl reductase family member 4. (237 aa) |
| | SFXN2 | Sidoreflexin. (322 aa) |
| | LYRM2 | LYR motif containing 2; Belongs to the complex I LYR family. (98 aa) |
| | CLPX | ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial. (633 aa) |
| | COQ10B | Coenzyme Q-binding protein COQ10 homolog B, mitochondrial. (242 aa) |
| | IDH3B | Isocitrate dehydrogenase [NAD] subunit, mitochondrial. (385 aa) |
| | MLYCD | Malonyl-CoA decarboxylase, mitochondrial. (493 aa) |
| | TMTC1 | Transmembrane O-mannosyltransferase targeting cadherins 1. (881 aa) |
| | EARS2 | Glutamyl-tRNA synthetase 2, mitochondrial; Belongs to the class-I aminoacyl-tRNA synthetase family. (534 aa) |
| | NDUFA5 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5. (116 aa) |
| | AMACR | Alpha-methylacyl-CoA racemase. (384 aa) |
| | NDUFA2 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2; Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. (99 aa) |
| | TIMM17A | Mitochondrial import inner membrane translocase subunit TIM17; Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane. (171 aa) |
| | NDUFV2 | NADH:ubiquinone oxidoreductase core subunit V2. (249 aa) |
| | PCCB | Uncharacterized protein. (568 aa) |
| | COX4I1 | Cytochrome c oxidase subunit 4I1. (169 aa) |
| | TXNRD2 | Thioredoxin reductase 2. (481 aa) |
| | OXCT1 | Succinyl-CoA:3-ketoacid-coenzyme A transferase; Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate. (534 aa) |
| | LOC100385103 | 10 kDa heat shock protein, mitochondrial; Belongs to the GroES chaperonin family. (102 aa) |
| | NDUFC1 | NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial. (76 aa) |
| | ENSCJAP00000056007 | Branched-chain-amino-acid aminotransferase; Belongs to the class-IV pyridoxal-phosphate-dependent aminotransferase family. (404 aa) |
| | COX8A | Cytochrome c oxidase subunit 8A. (69 aa) |
| | LOC100414136 | Protoheme IX farnesyltransferase, mitochondrial; Converts protoheme IX and farnesyl diphosphate to heme O. Belongs to the ubiA prenyltransferase family. (443 aa) |
| | THEM5 | Thioesterase superfamily member 5. (247 aa) |
| | FTH1 | Ferritin; Stores iron in a soluble, non-toxic, readily available form. Important for iron homeostasis. Iron is taken up in the ferrous form and deposited as ferric hydroxides after oxidation. (183 aa) |
| | MRPS30 | Mitochondrial ribosomal protein S30. (432 aa) |
| | ATP5MG | ATP synthase subunit; Mitochondrial membrane ATP synthase (F1F0 ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F1 - containing the extramembraneous catalytic core, and F0 - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F1 is coupled via a rotary mechanism of the central stalk subunits to proto [...] (103 aa) |
| | KYNU | Kynureninase; Catalyzes the cleavage of L-kynurenine (L-Kyn) and L-3- hydroxykynurenine (L-3OHKyn) into anthranilic acid (AA) and 3- hydroxyanthranilic acid (3-OHAA), respectively. Has a preference for the L-3-hydroxy form. Also has cysteine-conjugate-beta-lyase activity. Belongs to the kynureninase family. (424 aa) |
| | COMT | Catechol O-methyltransferase; Catalyzes the O-methylation, and thereby the inactivation, of catecholamine neurotransmitters and catechol hormones. (233 aa) |
| | ALAS1 | 5'-aminolevulinate synthase 1. (738 aa) |
| | PPARG | Peroxisome proliferator-activated receptor gamma; Nuclear receptor that binds peroxisome proliferators such as hypolipidemic drugs and fatty acids. Once activated by a ligand, the nuclear receptor binds to DNA specific PPAR response elements (PPRE) and modulates the transcription of its target genes, such as acyl-CoA oxidase. It therefore controls the peroxisomal beta-oxidation pathway of fatty acids. Key regulator of adipocyte differentiation and glucose homeostasis. ARF6 acts as a key regulator of the tissue-specific adipocyte P2 (aP2) enhancer. Acts as a critical regulator of gut ho [...] (505 aa) |
| | HSPA9 | Stress-70 protein, mitochondrial; Belongs to the heat shock protein 70 family. (679 aa) |
| | SUCLA2 | Succinate--CoA ligase [ADP-forming] subunit beta, mitochondrial; ATP-specific succinyl-CoA synthetase functions in the citric acid cycle (TCA), coupling the hydrolysis of succinyl-CoA to the synthesis of ATP and thus represents the only step of substrate-level phosphorylation in the TCA. The beta subunit provides nucleotide specificity of the enzyme and binds the substrate succinate, while the binding sites for coenzyme A and phosphate are found in the alpha subunit. (463 aa) |
| | NT5C3A | 5'-nucleotidase; Belongs to the pyrimidine 5'-nucleotidase family. (336 aa) |
| | ABCB10 | ATP-binding cassette sub-family B member 10, mitochondrial. (730 aa) |
| | AGXT2 | Alanine--glyoxylate aminotransferase 2; Belongs to the class-III pyridoxal-phosphate-dependent aminotransferase family. (517 aa) |
| | ALDH5A1 | Succinate-semialdehyde dehydrogenase; Belongs to the aldehyde dehydrogenase family. (535 aa) |
| | LOC100393688 | Creatine kinase, mitochondrial 1A (Predicted); Belongs to the ATP:guanido phosphotransferase family. (417 aa) |
| | COX7A1 | Uncharacterized protein. (80 aa) |
| | NDUFB9 | NADH:ubiquinone oxidoreductase subunit B9; Belongs to the complex I LYR family. (185 aa) |
| | PITRM1 | Pitrilysin metallopeptidase 1. (1038 aa) |
| | ABHD10 | Alpha/beta hydrolase domain-containing protein 10, mitochondrial isoform 1. (306 aa) |
| | NDUFS3 | NADH:ubiquinone oxidoreductase core subunit S3; Belongs to the complex I 30 kDa subunit family. (262 aa) |
| | PDHB | Pyruvate dehydrogenase E1 component subunit beta; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2. (359 aa) |
| | IMMT | MICOS complex subunit MIC60; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. (737 aa) |
| | ACSL5 | Acyl-CoA synthetase long chain family member 5. (750 aa) |
| | MIPEP | Mitochondrial intermediate peptidase. (706 aa) |
| | USMG5 | Up-regulated during skeletal muscle growth protein 5. (58 aa) |
| | SND1 | Staphylococcal nuclease domain-containing protein; Endonuclease that mediates miRNA decay of both protein-free and AGO2-loaded miRNAs. (910 aa) |
| | TXN | Thioredoxin; Participates in various redox reactions through the reversible oxidation of its active center dithiol to a disulfide and catalyzes dithiol-disulfide exchange reactions (By similarity). Plays a role in the reversible S-nitrosylation of cysteine residues in target proteins, and thereby contributes to the response to intracellular nitric oxide. Nitrosylates the active site Cys of CASP3 in response to nitric oxide (NO), and thereby inhibits caspase-3 activity. Induces the FOS/JUN AP-1 DNA binding activity in ionizing radiation (IR) cells through its oxidation/reduction status [...] (219 aa) |
| | HSD17B4 | Uncharacterized protein. (760 aa) |
| | LONP1 | Lon protease homolog, mitochondrial; ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial promoters and RNA in a single-stranded, site-specific, and strand- specific manner. May regulate mi [...] (960 aa) |
| | IMMP2L | Mitochondrial inner membrane protease subunit. (175 aa) |
| | SLC25A23 | Solute carrier family 25 member 23; Belongs to the mitochondrial carrier (TC 2.A.29) family. (468 aa) |
| | MDH2 | Malate dehydrogenase. (338 aa) |
| | RPL34 | Uncharacterized protein. (117 aa) |
| | AIFM3 | Apoptosis inducing factor mitochondria associated 3. (605 aa) |
| | DUS2 | tRNA-dihydrouridine(20) synthase [NAD(P)+]-like isoform 1. (493 aa) |
| | DRG2 | Developmentally-regulated GTP-binding protein 2. (364 aa) |
| | HAGH | Hydroxyacylglutathione hydrolase, mitochondrial; Thiolesterase that catalyzes the hydrolysis of S-D-lactoyl- glutathione to form glutathione and D-lactic acid. (308 aa) |
| | ATP5PD | ATP synthase subunit d, mitochondrial; Mitochondrial membrane ATP synthase (F(1)F(0) ATP synthase or Complex V) produces ATP from ADP in the presence of a proton gradient across the membrane which is generated by electron transport complexes of the respiratory chain. F-type ATPases consist of two structural domains, F(1) - containing the extramembraneous catalytic core, and F(0) - containing the membrane proton channel, linked together by a central stalk and a peripheral stalk. During catalysis, ATP synthesis in the catalytic domain of F(1) is coupled via a rotary mechanism of the cent [...] (161 aa) |
| | BDH1 | D-beta-hydroxybutyrate dehydrogenase, mitochondrial; Belongs to the short-chain dehydrogenases/reductases (SDR) family. (343 aa) |
| | VDAC1 | Voltage dependent anion channel 1. (357 aa) |
| | ATP5PB | ATP synthase subunit b, mitochondrial. (256 aa) |
| | UQCRQ | Ubiquinol-cytochrome c reductase complex III subunit VII. (82 aa) |
| | TOMM40 | Translocase of outer mitochondrial membrane 40. (361 aa) |
| | NDUFB5 | Uncharacterized protein. (189 aa) |
| | PXMP2 | Peroxisomal membrane protein 2; Belongs to the peroxisomal membrane protein PXMP2/4 family. (195 aa) |
| | GOT2 | Aspartate aminotransferase. (430 aa) |
| | COX7C | Cytochrome c oxidase subunit 7C. (63 aa) |
| | ALDH7A1 | Aldehyde dehydrogenase 7 family member A1; Belongs to the aldehyde dehydrogenase family. (513 aa) |
| | ETFA | Electron transfer flavoprotein subunit alpha. (333 aa) |
| | NDUFA7 | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7. (113 aa) |
| | PRODH | Proline dehydrogenase; Converts proline to delta-1-pyrroline-5-carboxylate. (600 aa) |
| | NAGS | N-acetylglutamate synthase. (534 aa) |
| | IREB2 | Iron-responsive element-binding protein 2; Belongs to the aconitase/IPM isomerase family. (964 aa) |
| | IDH3A | Isocitrate dehydrogenase [NAD] subunit, mitochondrial. (366 aa) |
| | DARS2 | Aspartyl-tRNA synthetase 2, mitochondrial. (645 aa) |
| | LOC100389097 | Cytochrome b-c1 complex subunit 2, mitochondrial. (453 aa) |
| | ATPAF2 | ATP synthase mitochondrial F1 complex assembly factor 2. (289 aa) |
| | FOXRED1 | FAD dependent oxidoreductase domain containing 1. (530 aa) |
| | LOC100395383 | ATP synthase subunit alpha; Produces ATP from ADP in the presence of a proton gradient across the membrane. (553 aa) |
| | HIBADH | 3-hydroxyisobutyrate dehydrogenase; Belongs to the HIBADH-related family. (336 aa) |
| | TDRKH | Tudor and KH domain containing isoform a (Predicted). (560 aa) |
| | SDHB | Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial; Iron-sulfur protein (IP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). (280 aa) |
| | NDUFB6 | Uncharacterized protein. (132 aa) |
| | ACO1 | Aconitase 1; Belongs to the aconitase/IPM isomerase family. (889 aa) |
| | SHMT2 | Serine hydroxymethyltransferase; Interconversion of serine and glycine. Belongs to the SHMT family. (504 aa) |
| | ACSL4 | Acyl-CoA synthetase long chain family member 4. (799 aa) |
| | SLC25A12 | Calcium-binding mitochondrial carrier protein Aralar1; Belongs to the mitochondrial carrier (TC 2.A.29) family. (678 aa) |
| | LDHA | L-lactate dehydrogenase; Belongs to the LDH/MDH superfamily. (361 aa) |
| | DDAH1 | N(G),N(G)-dimethylarginine dimethylaminohydrolase 1 isoform 1. (285 aa) |
| | OXR1 | Oxidation resistance 1. (883 aa) |
| | PINK1 | Serine/threonine-protein kinase PINK1, mitochondrial. (581 aa) |
| | PPM1K | Protein phosphatase, Mg2+/Mn2+ dependent 1K. (372 aa) |
| | TUFM | Elongation factor Tu; This protein promotes the GTP-dependent binding of aminoacyl- tRNA to the A-site of ribosomes during protein biosynthesis. (455 aa) |
| | NDUFA13 | Uncharacterized protein. (151 aa) |
| | PDHA1 | Pyruvate dehydrogenase E1 component subunit alpha; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). (426 aa) |
| | NDUFS2 | NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform 1; Belongs to the complex I 49 kDa subunit family. (463 aa) |
| | ACO2 | Aconitase 2. (805 aa) |
| | PRDX2 | Peroxiredoxin-2 isoform a. (198 aa) |
| | PDK3 | Protein-serine/threonine kinase. (424 aa) |
| | NDUFB7 | NADH:ubiquinone oxidoreductase subunit B7. (138 aa) |
| | RAB8B | RAB8B, member RAS oncogene family. (207 aa) |
| | NDUFB3 | NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3. (98 aa) |
| | UQCRC1 | Ubiquinol-cytochrome c reductase core protein 1. (491 aa) |
| | PARK7 | Protein DJ-1. (189 aa) |
| | MRPL42 | Uncharacterized protein. (154 aa) |
| | NME6 | Nucleoside diphosphate kinase. (194 aa) |
| | SLC25A3 | Solute carrier family 25 member 3; Belongs to the mitochondrial carrier (TC 2.A.29) family. (362 aa) |
| | ACP6 | Lysophosphatidic acid phosphatase type 6; Belongs to the histidine acid phosphatase family. (428 aa) |
| | CERK | Ceramide kinase. (448 aa) |
| | LOC100395443 | Cytochrome b-c1 complex subunit Rieske, mitochondrial; Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis. (274 aa) |
| | MRPL40 | 39S ribosomal protein L40, mitochondrial. (207 aa) |
| | PTRH2 | Peptidyl-tRNA hydrolase 2. (180 aa) |
| | VDAC2 | Voltage-dependent anion-selective channel protein 2 isoform 2. (294 aa) |
| | COMTD1 | Catechol O-methyltransferase domain-containing protein 1. (262 aa) |
| | SHMT1 | Serine hydroxymethyltransferase; Interconversion of serine and glycine. Belongs to the SHMT family. (483 aa) |
| | NDUFV1 | NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial; Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. (464 aa) |
| | TOMM70 | Mitochondrial import receptor subunit TOM70. (608 aa) |
| | VAMP1 | Vesicle associated membrane protein 1. (161 aa) |
| | DDX28 | DEAD-box helicase 28. (546 aa) |
| | LOC100403393 | Cytochrome b5 type B; Belongs to the cytochrome b5 family. (150 aa) |
| | HSDL1 | Hydroxysteroid dehydrogenase like 1; Belongs to the short-chain dehydrogenases/reductases (SDR) family. (478 aa) |
| | PNKD | PNKD metallo-beta-lactamase domain containing. (424 aa) |
| | MAP2 | Microtubule-associated protein. (1945 aa) |
| | SLC25A29 | Mitochondrial carnitine/acylcarnitine carrier protein CACL; Belongs to the mitochondrial carrier (TC 2.A.29) family. (303 aa) |
| | PPA2 | Pyrophosphatase (inorganic) 2. (345 aa) |
| | LOC100392617 | Cytochrome b5 heme-binding domain-containing protein; Belongs to the cytochrome b5 family. (279 aa) |
| | COX7B | Cytochrome c oxidase subunit 7B. (84 aa) |
| | QDPR | Uncharacterized protein. (248 aa) |
| | SLC25A4 | Solute carrier family 25 member 4; Belongs to the mitochondrial carrier (TC 2.A.29) family. (369 aa) |
| | TRMT10C | tRNA methyltransferase 10C, mitochondrial RNase P subunit. (424 aa) |
| | ALDH1L1 | Aldehyde dehydrogenase 1 family member L1; Belongs to the aldehyde dehydrogenase family. (944 aa) |
| | TOMM40-2 | Uncharacterized protein. (241 aa) |
| | GPX1 | Glutathione peroxidase 1; Protects the hemoglobin in erythrocytes from oxidative breakdown. (141 aa) |
| | NDUFS4 | NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial. (175 aa) |
